BACKGROUND Neuroendocrine tumors of the thymus (NETT) are a rare tumor entity of the anterior mediastinum. They belong to the category of foregut carcinoids and are often associated with the multiple endocrine neoplasia type 1 (MEN1) syndrome. Approximately 180 cases have been reported since their first description. NETT reveal an aggressive behavior leading to rapid local invasion and metastatic spread. An aggressive surgical approach may achieve long-term survival. METHODS Patients presenting from 1990 to 2005 at the Department of Surgery and the Department of Gastroenterology of the Philipps-University Marburg with neuroendocrine tumors were enrolled in a prospective database with a follow-up until 2005. Fifty MEN1-patients were enrolled in a study and screening program. These databases were retrospectively reviewed identifying all patients with NETT. The clinical features, therapeutical approaches and the outcome were analyzed. RESULTS Six patients were found with NETT, 4 patients suffered from metastases at the time of presentation. All patients were male, with a median age of 41.3 years at presentation. Four out of these 6 patients revealed MEN1 syndrome. All patients underwent tumor resection via sternotomy. Three patients underwent parathyreoidectomy and transcervical thymectomy before the NETT was diagnosed. Median survival was 53 months (range, 24-109). CONCLUSIONS Given a frequent association between MEN1 and NETT, all patients with NETT should be screened for MEN1. Since transcervical thymectomy does not prevent all MEN1 patients from developing NETT, existing surveillance guidelines for MEN1 should consider CT scan of the thorax on a regular basis.